Chapter 055. Immunologically Mediated Skin Diseases (Part 9)

Discoid lupus erythematosus.Violaceous, hyperpigmented, atrophic plaques, often with evidence of follicular plugging, which may result in scarring, are characteristic of discoid lupus erythematosus (also called chronic cutaneous lupus erythematosus).Scleroderma and MorpheaThe skin changes of scleroderma (Chap. 316) usually begin on the hands, feet, and face, with episodes of recurrent nonpitting edema. Sclerosis of the skin begins distally on the fingers (sclerodactyly) and spreads proximally, usually accompanied by resorption of bone of the fingertips, which may have punched out ulcers, stellate scars, or areas of hemorrhage (Fig. 55-7). The fingers may actually shrink in size and become sausage-shaped, and since...
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Chapter 055. Immunologically Mediated Skin Diseases (Part 9) Chapter 055. Immunologically Mediated Skin Diseases (Part 9)Figure 55-6 Discoid lupus erythematosus. Violaceous, hyperpigmented, atrophic plaques, often with evidence offollicular plugging, which may result in scarring, are characteristic of discoidlupus erythematosus (also called chronic cutaneous lupus erythematosus). Scleroderma and Morphea The skin changes of scleroderma (Chap. 316) usually begin on the hands,feet, and face, with episodes of recurrent nonpitting edema. Sclerosis of the skinbegins distally on the fingers (sclerodactyly) and spreads proximally, usuallyaccompanied by resorption of bone of the fingertips, which may have punched outulcers, stellate scars, or areas of hemorrhage (Fig. 55-7). The fingers may actuallyshrink in size and become sausage-shaped, and since the fingernails are usuallyunaffected, the nails may curve over the end of the fingertips. Periungual telangiectases are usually present, but periungual erythema israre. In advanced cases, the extremities show contractures and calcinosis cutis.Facial involvement includes a smooth, unwrinkled brow, taut skin over the nose,shrinkage of tissue around the mouth, and perioral radial furrowing (Fig. 55-8). Matlike telangiectases are often present, particularly on the face and hands.Involved skin feels indurated, smooth, and bound to underlying structures;hyperpigmentation and hypopigmentation are also often present. Raynaudsphenomenon, i.e., cold-induced blanching, cyanosis, and reactive hyperemia, ispresent in almost all patients and can precede development of scleroderma bymany years. The combination of calcinosis cutis, Raynauds phenomenon,esophageal dysmotility, sclerodactyly, and telangiectasia has been termed theCREST syndrome. Anticentromere antibodies have been reported in a very high percentage ofpatients with the CREST syndrome but in only a small minority of patients withscleroderma. Skin biopsy reveals thickening of the dermis and homogenization ofcollagen bundles. Direct immunofluorescence microscopy of lesional skin isusually negative. Figure 55-7Scleroderma showing acral sclerosis and focal digital ulcers.Figure 55-8